Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation

M Watanabe; N Yamamoto; N Ohkoshi; H Nagata; Y Kohno; A Hayashi; A Tamaoka; S Shoji

doi:10.1212/wnl.59.5.767

Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation

Neurology. 2002 Sep 10;59(5):767-9. doi: 10.1212/wnl.59.5.767.

Authors

M Watanabe¹, N Yamamoto, N Ohkoshi, H Nagata, Y Kohno, A Hayashi, A Tamaoka, S Shoji

Affiliation

¹ Department of Neurology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan. masa.wat@igaku.md.tsukuba.ac.jp

PMID: 12221176
DOI: 10.1212/wnl.59.5.767

Abstract

A patient with hereditary neuropathy presented with asymmetric distal weakness. On nerve biopsy, there was demyelination and onion-bulb formation, and molecular analysis revealed that the patient was heterozygous for an MPZ mutation. The patient improved with corticosteroid treatment.

Publication types

Case Reports
Review

MeSH terms

Adrenal Cortex Hormones / therapeutic use*
Adult
Biopsy
Female
Hereditary Sensory and Motor Neuropathy / drug therapy*
Hereditary Sensory and Motor Neuropathy / genetics*
Hereditary Sensory and Motor Neuropathy / pathology
Humans
Myelin P0 Protein / genetics*
Point Mutation*
Sural Nerve / pathology

Substances

Adrenal Cortex Hormones
Myelin P0 Protein